Terminal Complement Pathway Deficiency in an Adult Patient with Meningococcal Sepsis
The complement system is an essential part of our innate immune system. Three enzymatic activation pathways are described, all converging into a common terminal pathway which causes lysis of the target cell. Late complement deficiencies (LCDs) are typically diagnosed in children or adolescents with...
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| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
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Wiley
2022-01-01
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| Series: | Case Reports in Immunology |
| Online Access: | http://dx.doi.org/10.1155/2022/9057000 |
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| author | F. Staels W. Meersseman P. Stordeur K. Willekens S. Van Loo A. Corveleyn I. Meyts G. Meyfroidt R. Schrijvers |
| author_facet | F. Staels W. Meersseman P. Stordeur K. Willekens S. Van Loo A. Corveleyn I. Meyts G. Meyfroidt R. Schrijvers |
| author_sort | F. Staels |
| collection | DOAJ |
| description | The complement system is an essential part of our innate immune system. Three enzymatic activation pathways are described, all converging into a common terminal pathway which causes lysis of the target cell. Late complement deficiencies (LCDs) are typically diagnosed in children or adolescents with invasive meningococcal disease (IMD). However, IMD can also be a first manifestation in adulthood and should prompt for the evaluation of the LCD. We report the case of a young adult with IMD who was found to have a LCD, caused by a compound heterozygous mutation in C6. His vaccination status was optimized and prophylactic antibiotic treatment was initiated. By means of this case, we would like to raise awareness of underlying LCD in (young) adults presenting with IMD by N. meningitidis. Screening for complement deficiencies after IMD, followed by genetic testing, can be lifesaving and allows for genetic counselling. In addition, we discuss the diagnosis and treatment of LCD. |
| format | Article |
| id | doaj-art-180d69ce3c1a48d4b00de64016bfb810 |
| institution | Kabale University |
| issn | 2090-6617 |
| language | English |
| publishDate | 2022-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Immunology |
| spelling | doaj-art-180d69ce3c1a48d4b00de64016bfb8102025-02-03T01:06:50ZengWileyCase Reports in Immunology2090-66172022-01-01202210.1155/2022/9057000Terminal Complement Pathway Deficiency in an Adult Patient with Meningococcal SepsisF. Staels0W. Meersseman1P. Stordeur2K. Willekens3S. Van Loo4A. Corveleyn5I. Meyts6G. Meyfroidt7R. Schrijvers8Department of MicrobiologyDepartment of General Internal MedicineBelgian National Reference Center for the Complement SystemCenter for Human GeneticsCenter for Human GeneticsCenter for Human GeneticsDepartment of MicrobiologyDepartment of Intensive CareDepartment of MicrobiologyThe complement system is an essential part of our innate immune system. Three enzymatic activation pathways are described, all converging into a common terminal pathway which causes lysis of the target cell. Late complement deficiencies (LCDs) are typically diagnosed in children or adolescents with invasive meningococcal disease (IMD). However, IMD can also be a first manifestation in adulthood and should prompt for the evaluation of the LCD. We report the case of a young adult with IMD who was found to have a LCD, caused by a compound heterozygous mutation in C6. His vaccination status was optimized and prophylactic antibiotic treatment was initiated. By means of this case, we would like to raise awareness of underlying LCD in (young) adults presenting with IMD by N. meningitidis. Screening for complement deficiencies after IMD, followed by genetic testing, can be lifesaving and allows for genetic counselling. In addition, we discuss the diagnosis and treatment of LCD.http://dx.doi.org/10.1155/2022/9057000 |
| spellingShingle | F. Staels W. Meersseman P. Stordeur K. Willekens S. Van Loo A. Corveleyn I. Meyts G. Meyfroidt R. Schrijvers Terminal Complement Pathway Deficiency in an Adult Patient with Meningococcal Sepsis Case Reports in Immunology |
| title | Terminal Complement Pathway Deficiency in an Adult Patient with Meningococcal Sepsis |
| title_full | Terminal Complement Pathway Deficiency in an Adult Patient with Meningococcal Sepsis |
| title_fullStr | Terminal Complement Pathway Deficiency in an Adult Patient with Meningococcal Sepsis |
| title_full_unstemmed | Terminal Complement Pathway Deficiency in an Adult Patient with Meningococcal Sepsis |
| title_short | Terminal Complement Pathway Deficiency in an Adult Patient with Meningococcal Sepsis |
| title_sort | terminal complement pathway deficiency in an adult patient with meningococcal sepsis |
| url | http://dx.doi.org/10.1155/2022/9057000 |
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